Self-perceived Health Exceeds Societal Valuation Across Cardiac Neuromuscular Disease: Implications for Device Therapy Decision-making.

BACKGROUND: Cardiac complications are a leading cause of mortality in neuromuscular disease (NMD). Clinical assessment typically infers quality of life from functional impairment, which may overlook the disability paradox. We examined how patient-reported domain impairment, societal health valuation, and self-perceived health relate to one another in cardiac NMD. METHODS: We surveyed 156 patients (61% response rate) at a specialized cardiac NMD clinic using the EuroQol 5-Dimension 5-Level questionnaire. Three constructs were compared: domain impairment (5 domain levels); societal valuation (Index Score); and self-perceived health (Visual Analogue Scale [VAS]). Spearman correlations quantified associations; VAS was compared across mobility strata by Kruskal-Wallis testing. RESULTS: The cohort included 47% with cardiomyopathy and 28% with conduction disease. Ninety-one percent reported impairment in at least 1 domain, most often mobility (74%) and pain/discomfort (71%). VAS and Index Score were moderately correlated (ρ = 0.544, P < 0.001). VAS declined across mobility strata (81.5 to 61.1 from no problems to unable to walk; P < 0.001), but this 25% relative decline was far shallower than the 63% decline in Index Score (0.888-0.329). Mean VAS was 68.6 (standard deviation 20.6), lower than the Alberta population norm of 77.4 (Cohen d = 0.43) but modest given the burden of domain impairment. CONCLUSIONS: In cardiac NMD, self-perceived health declines less steeply than societal valuation as functional impairment worsens, consistent with psychological adaptation. Direct measurement of patient-reported well-being should inform shared decision-making around device therapies.